窄谱中波紫外线治疗早期蕈样肉芽肿9例疗效观察

目的:观察窄谱中波紫外线(NB-UVB)治疗早期蕈样肉芽肿的疗效及不良反应。方法:使用NB-UVB照射治疗早期蕈样肉芽肿患者9例,初始照射剂量均定为0.5J/cm2。结果:9例患者中完全缓解5例,部分缓解2例,好转2例,有效率为78%。平均照射次数22次,平均累积照射剂量31.09J/cm2。不良反应主要为轻度红斑和瘙痒,其发生率为33%。结论:NB-UVB照射治疗早期蕈样肉芽肿疗效好,不良反应少,操作简便易行,为早期蕈样肉芽肿的治疗提供了一种有效的方法。     

Bexarotene and narrowband ultraviolet B phototherapy combination treatment for mycosis fungoides

Combination therapy for mycosis fungoides (MF) has the potential to be synergistic, improve therapeutic efficacy and reduce toxicities. We present a patient with MF who improved on combination therapy with bexarotene and narrowband ultraviolet B (NB-UVB) therapy. The patient is an 81-year-old Caucasian male who initially presented with stage IB MF. After temporary improvement with NB-UVB phototherapy, he progressed to develop plaques and tumors. Psoralen and ultraviolet A therapy was contraindicated because of ophthalmologic disease. Addition of bexarotene 300 mg daily led to rapid clinical improvement in combination with NB-UVB. Interruption of NB-UVB during a prolonged hospitalization led to a clinical flare of lesions, despite continued treatment with bexarotene. Reinitiating NB-UVB was associated with clinical improvement. This report demonstrates that combination treatment with oral bexarotene and NB-UVB therapy may represent a safe alternative for the treatment of plaque-stage MF.     

Clinicopathological spectrum of mycosis fungoides

Cutaneous lymphomas represent a heterogeneous group of T-, NK- and B-cell neoplasms, with mycosis fungoides (MF) being the most common subtype. MF has a plethora of clinicopathological manifestations. Many variants of this lymphoma differ substantially from the 'classical' Alibert-Bazin disease and are therefore sometimes referred to as 'atypical' forms of the disease. This review addresses the whole clinicopathological spectrum of mycosis fungoides with respect to epidemiology, clinical, histopathological, immunophenotypic and genotypic features and the clinical course and prognosis of its variants: classical, erythrodermic, follicular, syringotropic, bullous/vesicular, granulomatous, poikilodermic, hypo- and hyperpigmented, unilesional, palmoplantar, hyperkeratotic/verrucous, vegetating/papillomatous, ichthyosiform, pigmented purpura-like, pustular and mucosal involvement in MF.     

Mycosis fungoides in a referral center in central Taiwan: A retrospective case series and literature review

Background/ObjectiveMycosis fungoides (MF) comprises the majority of cutaneous T-cell lymphomas (CTCLs). CTCLs associated with eosinophilia have a poor prognosis. Similar results were shown in white and black individuals with MF; however, the data on Asians is scant. In the past 10 years, few studies have provided profiles of the characteristics of MF patients in Taiwan. The purpose of this study was to investigate the demographic, clinicopathologic features, and prognosis of MF patients in Taiwan.MethodsA retrospective analysis was used to evaluate patients with MF in a referral center in central Taiwan covering a period of 16 years, from 1997 to 2013. The records of 22 Taiwanese patients with MF were reviewed for clinical, laboratory, and histopathologic data and evaluated by analysis of variance.ResultsThe male to female ratio was approximately 2:1. The average age at diagnosis was 44.8 years. One pediatric patient presented with hypopigmented MF, and the other 21 patients had typical clinical manifestations with patches-to-plaques, tumors or erythroderma. Common histopathologic features in over half of the patients included epidermotropism, atypical lymphocytes, vacuolar interface changes, and Pautrier's microabscesses. Treatment modalities, including skin-directed and systemic therapies, primarily depended on the clinical staging. Age 65 years or over (p = 0.004), and Stage IIB disease or higher (p = 0.026) were significant contributors to disease-specific mortality. There was no significant sex difference in overall survival. Of the 22 patients, 36.3% had blood eosinophilia. Blood eosinophilia was associated with Stage II disease or higher (p = 0.029) and an increased number of treatment types (p = 0.018), but not lactate dehydrogenase (LDH).ConclusionAge 65 years or over, Stage IIB disease or higher, and blood eosinophilia may be poor prognostic factors for Taiwanese patients with MF.     

Transformed mycosis fungoides: clinicopathological features and outcome

BACKGROUND: Transformation of mycosis fungoides (T-MF) occurs in 8-55% of MF patients. Its early histopathological diagnosis is of tremendous importance to better define the management and to establish the prognosis. Recent studies have demonstrated that advanced-stage MF at diagnosis of transformation is the predominant risk factor of poor outcome. The 5-year survival rates for stage IIB and IV MF are 26.9% and 10.6%, respectively. The prognostic value of the immunophenotypic characterization of the infiltrate has not been thoroughly studied in the literature. OBJECTIVES: To retrieve clinical, histological and immunophenotypic features of T-MF in our patient population and analyse their prognostic value. PATIENTS AND METHODS: A register-based retrospective study was performed including all patients with cutaneous T-cell lymphoma (CTCL) registered in our two departments from January 2000 to December 2005. Among 208 patients with CTCL, 17 patients with proven transformation of their MF were studied. Clinical features and staging as well as immunophenotypic and pathological findings at the time of the initial diagnosis of MF and of the diagnosis of T-MF were analysed. RESULTS: Our results, in accordance with previously published material, indicate that the main clinical prognostic factor in T-MF is the stage of the initial disease at the time of the transformation. Patients with stage IIB-IV MF have a poor prognosis. In our study, strong expression of CD30 is linked to a better prognosis. CONCLUSIONS: We believe that pathological and immunopathological documentation of progressive MF is important in order to identify T-MF early; however, the differential diagnosis is sometimes difficult. Aside from already acknowledged prognostic factors such as older age, advanced initial disease and short delay to transformation, the CD30 immunophenotype could be regarded as a useful additional prognostic marker in T-MF.     

Aggressive granulomatous mycosis fungoides with clinical pulmonary and thyroid involvement

We report a patient with granulomatous mycosis fungoides (MF) that progressed to a tumoral pattern and finally developed clinical pulmonary and thyroid involvement, despite multiple and intensive treatments. We emphasize the visceral involvement in this case, which was manifest as dyspnoea simulating pneumonia and by palpable thyroid nodules. These features are very unusual even in classic MF, and this is the first case in our knowledge of thyroid involvement in granulomatous MF.     

Cardiac involvement and molecular staging in a fatal case of mycosis fungoides

Polymerase chain reaction (PCR) amplification of T-cell receptor-gamma gene rearrangement was used for molecular staging in a case of primary cutaneous T-cell lymphoma (CTCL) with fatal evolution. Although initial evaluation was negative for systemic involvement, the patient died due to heart failure. Autopsy findings revealed lymphomatous myocardial infiltration, but other tissues and organs examined, including lymph nodes, liver, spleen, lung and bone marrow, appeared to be free of disease. Molecular analysis from frozen samples obtained during the initial evaluation, as well as paraffin-embedded material obtained during autopsy, revealed the presence of clonal rearranged bands in all tissues examined except the bone marrow. Subsequent hybridization of PCR products with a tumour-specific oligoprobe confirmed the PCR results, suggesting widespread dissemination of the lymphomatous process. The use of molecular analysis can add significant information about the extent of disease in patients with CTCL and may be helpful in the establishment of therapeutic options.     

Establishment of a mouse xenograft model for mycosis fungoides

Mycosis fungoides (MF) is the most frequent variant of cutaneous T-cell lymphomas (CTCLs). MF primarily involves the skin initially with patches and plaques. In later stages, cutaneous tumors develop and tumor cells may spread to lymph nodes and finally to visceral sites. Here, we describe an animal model for MF in immune-deficient nude mice, using the CTCL cell line MyLa. Subcutaneous transplantation of MyLa cells leads to the formation of cutaneous tumors in 80% of the mice (50/60 total). Spread of tumor cells to visceral sites was detected by immunohistochemistry and polymerase chain reaction (PCR)-based detection of specific T-cell receptor-gamma rearrangement. MyLa cells were found circulating in the blood, lymph nodes, and in blood vessels of heart, kidney, lung, and liver. In lung and liver tissue, tumor cells presented perivascular invasion, but no large secondary tumors developed. The nude mouse model described here will be a valuable test system for new therapeutic approaches for the treatment of MF and opens the unique opportunity to study the disease in vivo.     

Narrow-band ultraviolet therapy in early-stage mycosis fungoides: study on 20 patients

BACKGROUND: In recent years, narrow-band ultraviolet B (NB-UVB, 311-313 nm) has been found to be beneficial for early-stages mycosis fungoides (MF). The aim of this study is to investigate the effect of NB-UVB in 20 patients with early-stage MF. METHODS: Twenty patients (10 women and 10 men, mean age 54 +/- 22 years) with clinically and histologically confirmed MF were enrolled in the study. All of the patients had clinical stage I disease (T1 or T2, N0, M0) with cutaneous involvement, consisting of patch-stage disease of limited extent, in 50% of the cases (stage IA), and more widespread in the other 50% (stage IB). All the patients were treated with NB-UVB therapy until more than 95% clearance of the patient's skin lesions had occurred. RESULTS: A complete response was achieved in 90% of the cases after a mean of 29 +/- 14 treatments within a mean period of 4 months (range 1-8 months), with an average cumulative dose of 25 +/- 16.77 J/cm(2). In the follow-up period, relapse occurred after a mean period of 8 months (range 3-17 months), and then therapy was restarted. CONCLUSION: This study provides evidence that NB-UVB might be an efficient option for stage IA and IB MF patients.     

Effective remission of chidamide on treatment of advanced mycosis fungoides: An unusual case report

Mycosis fungoides (MF) and Sézary syndrome are the most common types of primary cutaneous T cell lymphoma, which primarily involves skin without curative treatment. We report a case of a 29‐year‐old female Chinese patient, who developed multiple cutaneous lesions gradually for 5 years. However, the patient could not tolerate pruritus and ulceration of cutaneous lesions, so she was admitted to hospital and diagnosed with advanced MF based on clinical manifestation, laboratory, and image results. Then she received four cycles of COPE regimen (cyclophosphamide, vincristine, prednisolone, and etoposide). Although the skin lesions shrinked and sense of pruritus alleviated quickly after the chemotherapy, the effective remission duration was not satisfactory. Therefore, the patient received gemcitabine and thalidomide for three cycles subsequently. Still the remission duration was not long lasting. After that, she took chidamide orally two times a week. By the time of 7 weeks, the patches almost diminished and the patient did not feel itching for almost 6 months. And, she did not have any adverse effect and had a better quality of life than the period of chemotherapy.     

Concurrent mycosis fungoides and intravascular large B-cell lymphoma in a single patient

Mycosis fungoides (MF) is an indolent, uncommon, non-Hodgkin T-cell lymphoma of the skin. It classically presents with patches, plaques, and tumors and may rarely show spread to internal organs or bone marrow. Up to 7.5% of MF patients may be diagnosed with a second malignancy. Intravascular large B-cell lymphoma (IVLBCL) is an exceedingly rare non-Hodgkin B-cell lymphoma characterized by predominant growth of large neoplastic cells in the lumina of blood vessels. This case presents with an unusual confluence of two rare diagnoses, MF and IVLBCL, made more remarkable by the presence of both diagnoses on a single skin biopsy sample.     

Efficacy of 8-methoxypsoralen vs. 5-methoxypsoralen plus ultraviolet A therapy in patients with mycosis fungoides

BACKGROUND: Psoralen plus ultraviolet (UV) A (PUVA) is the standard treatment for early stage mycosis fungoides (MF). When 8-methoxypsoralen (8-MOP) is used in PUVA therapy, it often produces intolerance reactions such as nausea, vomiting and headache. OBJECTIVES: To investigate whether 5-methoxypsoralen (5-MOP) is a safe and effective alternative to 8-MOP in PUVA therapy for MF. METHODS: A retrospective database search and chart review was done to identify patients with MF who received PUVA with either 5-MOP or 8-MOP as initial monotherapy at our institution. Between 1990 and 2004, 14 patients [seven men and seven women; mean age 70 years, range 51-82; National Cancer Institute disease stages IA (n = 6) and IB (n = 8)] received 5-MOP, and 24 patients [21 men and three women; mean age 58 years, range 28-89; disease stages IA (n = 11), IB (n = 12) and IIB (n = 1)] received 8-MOP. RESULTS: Twelve of 14 patients (86%) in the 5-MOP group and 22 of 24 (92%) in the 8-MOP group had a complete response to PUVA. These two subgroups of complete responders did not differ significantly in terms of PUVA therapy duration, number of treatments or cumulative UVA dose. They also did not differ significantly in terms of relapse-free rate [8% (one of 12) vs. 23% (five of 22)] or time to relapse [17 months (range 4-31) vs. 14 months (range 4-33)]. Moreover, PUVA maintenance therapy with either 5-MOP or 8-MOP in a subset of patients [26% (nine of 34)] did not affect long-term relapse-free status either. CONCLUSIONS: 5-MOP and 8-MOP have comparable therapeutic efficacy when used in PUVA therapy for MF.     

Nitrogen mustard gel-induced inflammation triggers lymphomatoid papulosis in patients with mycosis fungoides

Lymphomatoid papulosis (LyP) is a paraneoplastic primary cutaneous CD30⁺ lymphoproliferative disorder (LPD) that has been associated with malignant lymphomas, most commonly mycosis fungoides (MF). We observed 10 patients with MF who developed severe inflammation after using nitrogen-mustard (NM) gel from 1 to 8 months and who developed LyP. We hypothesized that NM gel produced local inflammation, which induced CD30 expression in malignant T cells in situ leading to the appearance of LyP papules. The high frequency of induction of LyP lesions in patients with severe inflammation while on treatment with NM gel suggests an association between inflammatory stimuli and development of LyP. Our observation provides insight into the pathogenesis of CD30⁺ LPD.